Wilms tumour and renal malignancies: Viva

Branching clinical structured oral on Wilms tumour and the paediatric renal malignancies, covering the recognition of the painless abdominal mass with the haematuria and the hypertension, the peak age of three to four years, the syndromic predisposition of the WAGR, the Beckwith-Wiedemann and the Denys-Drash, the first-line ultrasound with the Doppler of the cava, the Children's Oncology Group do-not-biopsy strategy against the SIOP preoperative chemotherapy, the risk-adapted surgery and chemotherapy, and the classic pitfalls around the tumour spill and the missed tumour thrombus.

branching clinical structured oral4 min readVerification in progress

Prompt

A three-year-old girl is brought in after her mother felt a lump in her abdomen at the bath time. She is otherwise well. The examination shows a smooth, firm, non-tender mass in the right flank that does not cross the midline, and the blood pressure is at the ninety-fifth percentile. The examiner asks how you frame the problem, what imaging you arrange, how you confirm and stage the diagnosis, and how you would counsel the family across the different tumour possibilities.

This is a branching oral built to probe the reasoning that holds the histology and the staging at the centre, and to expose the candidate who has memorised the headline without the corners. The questions escalate from the framing to the imaging, the staging, and the definitive management, with the deliberate probes into the pitfalls of the tumour spill and the missed thrombus. [1]

Opening question: framing the problem

The examiner opens with the history and the examination and asks: how do you frame this problem in a single sentence, and what is your first priority? [1]

A strong answer names the renal tumour, states that the painless abdominal mass that does not cross the midline in a three-year-old is a Wilms tumour until the imaging settles it, and identifies the hypertension as the accompanying feature. The first priority is the imaging that confirms the renal origin and excludes the tumour thrombus, and the avoidance of the biopsy. [4]

Model answer. This child has a renal tumour, almost certainly a Wilms tumour, until the imaging settles it. The smooth, firm, flank mass that does not cross the midline in a three-year-old, with the hypertension, is the classic presentation. My first priority is the ultrasound with the Doppler of the cava, the staging, and the urgent referral to the specialist centre, with the biopsy avoided in the typical case. [1]

Probe one: the imaging and the staging

The examiner presses: what imaging do you arrange, and why do you image the cava before the surgery? [9]

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References6Show ledger

[1]Spreafico F, Fernandez CV, Brok J Wilms tumour Nat Rev Dis Primers, 2021.PMID 34650095
[4]Dome JS, Mullen EA, Dix DB Impact of the First Generation of Children's Oncology Group Clinical Trials on Clinical Practice for Wilms Tumor J Natl Compr Canc Netw, 2021.PMID 34416705
[5]Graf N, Tournade MF, de Kraker J The role of preoperative chemotherapy in the management of Wilms' tumor. The SIOP studies Urol Clin North Am, 2000.PMID 10985144
[6]Kalish JM, Becktell KD, Bougeard G Update on Surveillance for Wilms Tumor and Hepatoblastoma in Beckwith-Wiedemann Syndrome and Other Predisposition Syndromes Clin Cancer Res, 2024.PMID 39320341
[8]Gadd S, Huff V, Walz AL A Children's Oncology Group and TARGET initiative exploring the genetic landscape of Wilms tumor Nat Genet, 2017.PMID 28825729
[9]Benedetti DJ, Varela CR, Renfro LA Treatment of children with favorable histology Wilms tumor with extrapulmonary metastases: a report from the COG studies AREN0533 and AREN03B2 and NWTSG study NWTS-5 Cancer, 2024.PMID 37933882